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doi:10.1111/jog.12377
J. Obstet. Gynaecol. Res. Vol. 40, No. 6: 1823–1827, June 2014
Primary retroperitoneal Müllerian adenocarcinoma arising from endometriosis Kei Tanaka, Yoichi Kobayashi, Hiromi Shibuya, Yoshiko Nishigaya, Mai Momomura, Hironori Matsumoto and Mitsutoshi Iwashita Department of Obstetrics and Gynecology, Kyorin University School of Medicine, Mitaka, Tokyo, Japan
Abstract Primary retroperitoneal Müllerian adenocarcinoma (PRMA) is an extremely rare tumor and the cause remains unknown. We report a case of PRMA arising from endometriosis. A 52-year-old woman with a history of malignant lymphoma underwent a follow-up computed tomography scan, which revealed a retroperitoneal tumor. Immunohistochemical analysis of tumor resected during laparoscopic surgery showed adenocarcinoma positive for cytokeratin 7 and negative for cytokeratin 20. The patient had undergone hysterectomy and bilateral salpingo-oophorectomy 14 years ago for myoma uteri and endometrial cysts and was treated with estrogen-replacement therapy. The size of the tumor increased and laparotomy was performed. Histopathological examination showed adenocarcinoma resembling endometrial adenocarcinoma, which stained positive for cancer antigen 125, cancer antigen 19-9, estrogen receptor, and progesterone receptor immunohistochemically. The focus of the endometriosis was found at the edge of the tumor, and the stromal cells around the tumor cells were CD10 positive. The patient was diagnosed as having PRMA arising from endometriosis, and treated with adjuvant chemotherapy. Key words: endometrioid carcinoma, endometriosis, estrogen-replacement therapy, Müllerian carcinoma, retroperitoneum.
Introduction
Case Report
Primary retroperitoneal Müllerian adenocarcinoma (PRMA) is an extremely rare tumor. Malignant Müllerian tumors usually arise in the uterine corpus; however, a few cases of origin from extragenital sites, such as the peritoneum, pelvic wall, subdiaphragmatic surface, and omentum, have been reported,1–3 among which the retroperitoneum is one of the rarest sites. Although the cause of extragenital malignant Müllerian tumors remains unknown, endometriosisorigin and secondary-Müllerian-system theories have been proposed.1,4 We report a case of PRMA, which was speculated to have arisen from endometriosis.
A 52-year-old nulliparous woman began to notice urinary frequency and experienced post-void residual urine for a year. She had a history of malignant lymphoma and had received chemotherapy (rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin, predonisone [R-CHOP]) at 49 years of age. She underwent a follow-up contrast-enhanced computed tomography (CT) scan, which revealed a 20-mm retroperitoneal cystic mass with an enhancing component on the left side of the bladder (Fig. 1). The CT image also showed thickening of the bladder wall adjacent to the tumor and an elevation of fat tissue density around
Received: October 10 2013. Accepted: December 10 2013. Reprint request to: Dr Yoichi Kobayashi, Kyorin University School of Medicine, Department of Obstetrics and Gynecology, Shinkawa 6-20-2, Mitaka, Tokyo 181-8611, Japan. Email: [email protected]
© 2014 The Authors Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology
1823
K. Tanaka et al.
(a)
(b)
Figure 1 (a) Contrast-enhanced computed tomography scan showing a 20-mm retroperitoneal cystic mass on the left side of the bladder. (b) Intraoperative findings on the laparoscopic surgery showing the tumor (arrow) in the retroperitoneal space, which firmly adhered to the bladder (B) and the sigmoid colon (S).
(a)
(c)
(b)
(d)
the tumor. Positron emission tomography revealed fluorodeoxyglucose accumulation in the retroperitoneal tumor. Subsequently, the tumor was partially resected by laparoscopic surgery for the purpose of diagnosis (Fig. 1). The tumor cells were cuboidal cells with atypical nuclei, adenocarcinoma, forming a tubular and cribriform pattern. Immunohistochemical analysis revealed that the surgical specimen was positive for cytokeratin (CK)7 and negative for CK20. Laboratory tests for tumor markers after surgery showed the following results: cancer antigen (CA)125, 50.2 IU/ml; CA19-9, 76.7 IU/ml; and CEA, 1.7 ng/ml. Fourteen years ago, the patient had undergone total abdominal hysterectomy and bilateral salpingooophorectomy for myoma uteri and endometrial cysts followed by estrogen-replacement therapy. A pathology specimen from the previous surgery was reviewed again, and the diagnosis of ovarian endometriosis and uterine leiomyoma with no evidence of malignancy
1824
Figure 2 (a) Gross findings of the retroperitoneal tumor showing a yellowish solid mass. (b) Microscopic findings of the retroperitoneal tumor showing irregular tubular columnar cells with enlarged nuclei, which resemble an endometrioid adenocarcinoma (hematoxylin– eosin [HE] ×40). (c,d) Ectopic benign endometrial tissue was present adjacent to the tumor cells. (c) HE ×4. (d) HE ×20.
was ascertained. Subsequently, the size of the tumor increased to 30 mm on contrast-enhanced CT 2 months after the laparoscopic surgery, following which laparotomy was performed for retroperitoneal tumorectomy, left ureterectomy, and partial cystectomy. The resected tumor was a yellowish solid mass without a clear border and measured 40 × 37 × 29 mm. On microscopic examination, the tumor cells were tubular, cribriform, and partly columnar with enlarged irregular nuclei and cystic chromatin arranged in a trabecular pattern, which resembled the appearance of an endometrioid adenocarcinoma (Fig. 2). The cytoplasm was eosinophilic and intraluminal mucus was positive for periodic acid–Schiff and Alcian-blue. Tumor cells were immunohistochemically reactive towards CK7, CA125, CA19-9, estrogen receptor (ER), progesterone receptor (PR), and PAX8, while they were negative for CK20 and CDX2. Stromal cells
© 2014 The Authors Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology
Retroperitoneal Müllerian tumor
(a)
(b)
(c)
(d)
(e)
Figure 3 Immunohistochemical examinations of the tumor cells showed positivity for (a) cytokeratin (CK) 7, (b) negativity for CK20 and positivity for (c) cancer antigen (CA)19-9 and (d) CA125. (e) The stromal cells around the tumor cells showed immunoreactivity towards CD10.
around the tumor cells were positive for cluster of differentiation (CD)10 (Fig. 3). Marked lymphovascular infiltration and invasion around the left ureter extending into lamina propria of the bladder was noted. Ectopic endometrial tissue was observed at the edge of the tumor (Fig. 2). On the basis of these findings, the patient was diagnosed with PRMA arising from endometriosis. Brush cytology examination of a vaginal stump performed a month after the laparotomy was positive for adenocarcinoma, following which she received adjuvant chemotherapy. The patient has not demonstrated any evidence of recurrence for the last 3 months.
Discussion In this case report, we present our experience in diagnosing and managing a case of PRMA arising from
endometriosis. Microscopic findings of the tumor showed that the adenocarcinoma resembled an endometrioid adenocarcinoma, which was positive for CK7, CA125, and CA19-9 and negative for CK20, implicating the Müllerian duct as the source of origin. No evidence of malignancy was found in the ovaries or uterus resected in the previous surgery, and no evidence of primary malignancy in the other organs was observed in the radiological examinations. These observations indicated that the tumor originated primarily from the retroperitoneum and was not a metastatic tumor. Increased risk of solid cancers among the survivors of non-Hodgkin’s lymphoma has been reported.5 Our patient had a history of chemotherapy (R-CHOP) for non-Hodgkin’s lymphoma 3 years ago. To our knowledge, the present case represents the first report of a PRMA after chemotherapy for malignant lymphoma.
© 2014 The Authors Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology
1825
K. Tanaka et al.
Microscopic examination showed coexistence of endometriosis with the tumor. Stromal cells around the tumor cells were positive for CD10, which is usually expressed in endometrial stromal cells of the uterus. As confirmed in a specimen obtained from the previous surgery, the patient had a history of endometrial ovarian cysts. Extraovarian cancers arising from endometriosis are more likely to occur in postmenopausal women and are thought to be the result of hormone-replacement therapy.6–8 Our patient had both artificial menopause by surgery and a history of estrogen-replacement therapy. Therefore, one can speculate that the present case of PRMA originated from malignant transformation of endometriosis. The exact origin of extragenital malignant Müllerian tumors is unknown. Two major hypotheses, the secondary-Müllerian-system and the endometriosisorigin theory, have been proposed. The first hypothesis is based on the existence of a ‘secondary Müllerian system’, which was discussed by Lauchlan.4 According to this hypothesis, the coelomic epithelium covering the female peritoneal surface has the potential for Müllerian differentiation, a potential shared by the underlying ‘sub-coelomic mesenchyme’, which may be the origin of the extragenital Müllerian-type neoplasms arising in the retroperitoneum. The second hypothesis assumes that the presence of endometriosis is a possible source of origin for PRMA.1 There are numerous published reports of extragonadal malignancies arising from endometriosis.9,10 Malignant transformation of endometriosis was first described by Sampson in 1925; he proposed the original criteria for carcinomatous development in endometriosis: (i) clear examples of endometriosis present in close proximity to the tumor; (ii) no other primary site found; and (iii) histological appearance suggests a possible origin from endometriosis.11 Our case satisfied all three criteria, which supported the endometriosis-origin theory of PRMA. Herein, we report a case of PRMA arising from endometriosis. Brooks and Wheeler first reported a case of clear cell carcinoma arising from endometriosis of the retroperitoneum.12 In their case, a middleaged woman also received estrogen-replacement therapy after hysterectomy and bilateral salpingooophorectomy and microscopically the tumor was composed of a papillary adenocarcinoma with hobnail cells. Benign endometrial glands and stroma were scattered directly beneath the tumor and atypical cells were seen in some of the foci of endometriosis. The tumor cells of our case were predominantly tubular and cribriform, not papillary, and resembled the
1826
appearance of an endometrioid adenocarcinoma. Although atypical cells were not detected, coexistence of ectopic endometrial tissue with the tumor was confirmed histologically. To our knowledge, our case is the second case of histologically proven endometriosisorigin PRMA. The pathophysiology of PRMA is diverse and previous reports have shown other histological types of PRMA. Some authors reported the papillary serous adenocarcinoma of the retroperitoneum with the microscopic finding of typical psammoma bodies.13,14 Ulbright et al. presented a pre-pubertal female case of retroperitoneal serous papillary adenocarcinoma, which they believed originated from metaplasia of mesothelium.13 Lawrence et al. described the relatively large cystic mucinous tumor of the retroperitoneum exhibiting a varying degree of differentiation, from benign mucinous cyst adenoma to papillary mucinous cyst adenocarcinoma.15 These cases suggest that not all PRMA originate from endometriosis. PRMA is an extremely rare tumor, and accurate diagnosis and investigation of more cases are essential to gain a better understanding.
Disclosure The authors have no conflicts of interest, sources of financial support, corporate involvement, or patent holdings to disclose.
References 1. Chumas JC, Thanning L, Mann WJ. Malignant mixed mullerian tumor arising in extragenital endometriosis: A report of case and review of the literature. Gynecol Oncol 1986; 23: 227–233. 2. Chen KTK, Wolk RW. Extragenital malignant mixed Mullerian tumor. Gynecol Oncol 1988; 30: 422–426. 3. Garde JR, Jones MA, McAfee R et al. Extragenital malignant mixed mullerian tumor: Review of the literature. Gynecol Oncol 1991; 43: 186–190. 4. Lauchlan SC. The secondary mullerian system revisited. Int J Gynecol Pathol 1994; 13 (1): 73–79. 5. Pirani M, Marcheselli R, Marcheselli L, Bari A, Federico M, Sacchi S. Risk for second malignancies in non-Hodgkin’s lymphoma survivors: A meta-analysis. Ann Oncol 2011; 22: 1845– 1858. 6. Gucer F, Pieber D, Arikan F. Malignancy arising in extraovarian endometriosis during estrogen stimulation. Eur J Gynaelcol Oncol 1998; 19: 39–41. 7. Lavery S, Gillmer M. Malignant transformation of residual endometriosis in women on unopposed oestrogen hormone replacement therapy. Br J Obstet Gynaecol 2001; 108: 1106– 1107.
© 2014 The Authors Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology
Retroperitoneal Müllerian tumor
8. Modesitt SC, Tortolero-Luna G, Robinson JB, Gershenson DM, Wolf JK. Ovarian and extraovarian endometriosisassociated cancer. Obstet Gynecol 2002; 100 (4): 788–795. 9. Heaps JM, Nieberg RK, Berek JS. Malignant neoplasms arising in endometriosis. Obstet Gynecol 1990; 75: 1023– 1028. 10. Han AC, Hovenden S, Rosenblum NG et al. Adenocarcinoma arising in extragonadal endometriosis: An immunohistochemical study. Cancer 1998; 83: 1163–1169. 11. Sampson JA. Endometrial carcinoma of the ovary, arising in endometrial tissue in that organ. Arch Surg 1925; 10: 1– 72.
12. Brooks JJ, Wheeler JE. Malignancy arising in extragonadal endometriosis. A case report and summary of the world literature. Cancer 1977; 40: 3065–3073. 13. Ulbright TM, Morley DJ, Roth LM, Berkow RL. Papillary serous carcinoma of the retroperitoneum. Am J Clin Pathol 1983; 79: 633–637. 14. Iura A, Sasajima Y, Katsumata N et al. Serous adenocarcinoma of the retroperitoneum, as a type of multifocal mullerian carcinoma. Int J Clin Oncol 2009; 14: 254– 257. 15. Roth LM, Ehrlich CE. Mucinous cystadenocarcinoma of the retroperitoneum. Obstet Gynecol 1977; 49: 486–488.
© 2014 The Authors Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology
1827
doi:10.1111/jog.12377
J. Obstet. Gynaecol. Res. Vol. 40, No. 6: 1823–1827, June 2014
Primary retroperitoneal Müllerian adenocarcinoma arising from endometriosis Kei Tanaka, Yoichi Kobayashi, Hiromi Shibuya, Yoshiko Nishigaya, Mai Momomura, Hironori Matsumoto and Mitsutoshi Iwashita Department of Obstetrics and Gynecology, Kyorin University School of Medicine, Mitaka, Tokyo, Japan
Abstract Primary retroperitoneal Müllerian adenocarcinoma (PRMA) is an extremely rare tumor and the cause remains unknown. We report a case of PRMA arising from endometriosis. A 52-year-old woman with a history of malignant lymphoma underwent a follow-up computed tomography scan, which revealed a retroperitoneal tumor. Immunohistochemical analysis of tumor resected during laparoscopic surgery showed adenocarcinoma positive for cytokeratin 7 and negative for cytokeratin 20. The patient had undergone hysterectomy and bilateral salpingo-oophorectomy 14 years ago for myoma uteri and endometrial cysts and was treated with estrogen-replacement therapy. The size of the tumor increased and laparotomy was performed. Histopathological examination showed adenocarcinoma resembling endometrial adenocarcinoma, which stained positive for cancer antigen 125, cancer antigen 19-9, estrogen receptor, and progesterone receptor immunohistochemically. The focus of the endometriosis was found at the edge of the tumor, and the stromal cells around the tumor cells were CD10 positive. The patient was diagnosed as having PRMA arising from endometriosis, and treated with adjuvant chemotherapy. Key words: endometrioid carcinoma, endometriosis, estrogen-replacement therapy, Müllerian carcinoma, retroperitoneum.
Introduction
Case Report
Primary retroperitoneal Müllerian adenocarcinoma (PRMA) is an extremely rare tumor. Malignant Müllerian tumors usually arise in the uterine corpus; however, a few cases of origin from extragenital sites, such as the peritoneum, pelvic wall, subdiaphragmatic surface, and omentum, have been reported,1–3 among which the retroperitoneum is one of the rarest sites. Although the cause of extragenital malignant Müllerian tumors remains unknown, endometriosisorigin and secondary-Müllerian-system theories have been proposed.1,4 We report a case of PRMA, which was speculated to have arisen from endometriosis.
A 52-year-old nulliparous woman began to notice urinary frequency and experienced post-void residual urine for a year. She had a history of malignant lymphoma and had received chemotherapy (rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin, predonisone [R-CHOP]) at 49 years of age. She underwent a follow-up contrast-enhanced computed tomography (CT) scan, which revealed a 20-mm retroperitoneal cystic mass with an enhancing component on the left side of the bladder (Fig. 1). The CT image also showed thickening of the bladder wall adjacent to the tumor and an elevation of fat tissue density around
Received: October 10 2013. Accepted: December 10 2013. Reprint request to: Dr Yoichi Kobayashi, Kyorin University School of Medicine, Department of Obstetrics and Gynecology, Shinkawa 6-20-2, Mitaka, Tokyo 181-8611, Japan. Email: [email protected]
© 2014 The Authors Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology
1823
K. Tanaka et al.
(a)
(b)
Figure 1 (a) Contrast-enhanced computed tomography scan showing a 20-mm retroperitoneal cystic mass on the left side of the bladder. (b) Intraoperative findings on the laparoscopic surgery showing the tumor (arrow) in the retroperitoneal space, which firmly adhered to the bladder (B) and the sigmoid colon (S).
(a)
(c)
(b)
(d)
the tumor. Positron emission tomography revealed fluorodeoxyglucose accumulation in the retroperitoneal tumor. Subsequently, the tumor was partially resected by laparoscopic surgery for the purpose of diagnosis (Fig. 1). The tumor cells were cuboidal cells with atypical nuclei, adenocarcinoma, forming a tubular and cribriform pattern. Immunohistochemical analysis revealed that the surgical specimen was positive for cytokeratin (CK)7 and negative for CK20. Laboratory tests for tumor markers after surgery showed the following results: cancer antigen (CA)125, 50.2 IU/ml; CA19-9, 76.7 IU/ml; and CEA, 1.7 ng/ml. Fourteen years ago, the patient had undergone total abdominal hysterectomy and bilateral salpingooophorectomy for myoma uteri and endometrial cysts followed by estrogen-replacement therapy. A pathology specimen from the previous surgery was reviewed again, and the diagnosis of ovarian endometriosis and uterine leiomyoma with no evidence of malignancy
1824
Figure 2 (a) Gross findings of the retroperitoneal tumor showing a yellowish solid mass. (b) Microscopic findings of the retroperitoneal tumor showing irregular tubular columnar cells with enlarged nuclei, which resemble an endometrioid adenocarcinoma (hematoxylin– eosin [HE] ×40). (c,d) Ectopic benign endometrial tissue was present adjacent to the tumor cells. (c) HE ×4. (d) HE ×20.
was ascertained. Subsequently, the size of the tumor increased to 30 mm on contrast-enhanced CT 2 months after the laparoscopic surgery, following which laparotomy was performed for retroperitoneal tumorectomy, left ureterectomy, and partial cystectomy. The resected tumor was a yellowish solid mass without a clear border and measured 40 × 37 × 29 mm. On microscopic examination, the tumor cells were tubular, cribriform, and partly columnar with enlarged irregular nuclei and cystic chromatin arranged in a trabecular pattern, which resembled the appearance of an endometrioid adenocarcinoma (Fig. 2). The cytoplasm was eosinophilic and intraluminal mucus was positive for periodic acid–Schiff and Alcian-blue. Tumor cells were immunohistochemically reactive towards CK7, CA125, CA19-9, estrogen receptor (ER), progesterone receptor (PR), and PAX8, while they were negative for CK20 and CDX2. Stromal cells
© 2014 The Authors Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology
Retroperitoneal Müllerian tumor
(a)
(b)
(c)
(d)
(e)
Figure 3 Immunohistochemical examinations of the tumor cells showed positivity for (a) cytokeratin (CK) 7, (b) negativity for CK20 and positivity for (c) cancer antigen (CA)19-9 and (d) CA125. (e) The stromal cells around the tumor cells showed immunoreactivity towards CD10.
around the tumor cells were positive for cluster of differentiation (CD)10 (Fig. 3). Marked lymphovascular infiltration and invasion around the left ureter extending into lamina propria of the bladder was noted. Ectopic endometrial tissue was observed at the edge of the tumor (Fig. 2). On the basis of these findings, the patient was diagnosed with PRMA arising from endometriosis. Brush cytology examination of a vaginal stump performed a month after the laparotomy was positive for adenocarcinoma, following which she received adjuvant chemotherapy. The patient has not demonstrated any evidence of recurrence for the last 3 months.
Discussion In this case report, we present our experience in diagnosing and managing a case of PRMA arising from
endometriosis. Microscopic findings of the tumor showed that the adenocarcinoma resembled an endometrioid adenocarcinoma, which was positive for CK7, CA125, and CA19-9 and negative for CK20, implicating the Müllerian duct as the source of origin. No evidence of malignancy was found in the ovaries or uterus resected in the previous surgery, and no evidence of primary malignancy in the other organs was observed in the radiological examinations. These observations indicated that the tumor originated primarily from the retroperitoneum and was not a metastatic tumor. Increased risk of solid cancers among the survivors of non-Hodgkin’s lymphoma has been reported.5 Our patient had a history of chemotherapy (R-CHOP) for non-Hodgkin’s lymphoma 3 years ago. To our knowledge, the present case represents the first report of a PRMA after chemotherapy for malignant lymphoma.
© 2014 The Authors Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology
1825
K. Tanaka et al.
Microscopic examination showed coexistence of endometriosis with the tumor. Stromal cells around the tumor cells were positive for CD10, which is usually expressed in endometrial stromal cells of the uterus. As confirmed in a specimen obtained from the previous surgery, the patient had a history of endometrial ovarian cysts. Extraovarian cancers arising from endometriosis are more likely to occur in postmenopausal women and are thought to be the result of hormone-replacement therapy.6–8 Our patient had both artificial menopause by surgery and a history of estrogen-replacement therapy. Therefore, one can speculate that the present case of PRMA originated from malignant transformation of endometriosis. The exact origin of extragenital malignant Müllerian tumors is unknown. Two major hypotheses, the secondary-Müllerian-system and the endometriosisorigin theory, have been proposed. The first hypothesis is based on the existence of a ‘secondary Müllerian system’, which was discussed by Lauchlan.4 According to this hypothesis, the coelomic epithelium covering the female peritoneal surface has the potential for Müllerian differentiation, a potential shared by the underlying ‘sub-coelomic mesenchyme’, which may be the origin of the extragenital Müllerian-type neoplasms arising in the retroperitoneum. The second hypothesis assumes that the presence of endometriosis is a possible source of origin for PRMA.1 There are numerous published reports of extragonadal malignancies arising from endometriosis.9,10 Malignant transformation of endometriosis was first described by Sampson in 1925; he proposed the original criteria for carcinomatous development in endometriosis: (i) clear examples of endometriosis present in close proximity to the tumor; (ii) no other primary site found; and (iii) histological appearance suggests a possible origin from endometriosis.11 Our case satisfied all three criteria, which supported the endometriosis-origin theory of PRMA. Herein, we report a case of PRMA arising from endometriosis. Brooks and Wheeler first reported a case of clear cell carcinoma arising from endometriosis of the retroperitoneum.12 In their case, a middleaged woman also received estrogen-replacement therapy after hysterectomy and bilateral salpingooophorectomy and microscopically the tumor was composed of a papillary adenocarcinoma with hobnail cells. Benign endometrial glands and stroma were scattered directly beneath the tumor and atypical cells were seen in some of the foci of endometriosis. The tumor cells of our case were predominantly tubular and cribriform, not papillary, and resembled the
1826
appearance of an endometrioid adenocarcinoma. Although atypical cells were not detected, coexistence of ectopic endometrial tissue with the tumor was confirmed histologically. To our knowledge, our case is the second case of histologically proven endometriosisorigin PRMA. The pathophysiology of PRMA is diverse and previous reports have shown other histological types of PRMA. Some authors reported the papillary serous adenocarcinoma of the retroperitoneum with the microscopic finding of typical psammoma bodies.13,14 Ulbright et al. presented a pre-pubertal female case of retroperitoneal serous papillary adenocarcinoma, which they believed originated from metaplasia of mesothelium.13 Lawrence et al. described the relatively large cystic mucinous tumor of the retroperitoneum exhibiting a varying degree of differentiation, from benign mucinous cyst adenoma to papillary mucinous cyst adenocarcinoma.15 These cases suggest that not all PRMA originate from endometriosis. PRMA is an extremely rare tumor, and accurate diagnosis and investigation of more cases are essential to gain a better understanding.
Disclosure The authors have no conflicts of interest, sources of financial support, corporate involvement, or patent holdings to disclose.
References 1. Chumas JC, Thanning L, Mann WJ. Malignant mixed mullerian tumor arising in extragenital endometriosis: A report of case and review of the literature. Gynecol Oncol 1986; 23: 227–233. 2. Chen KTK, Wolk RW. Extragenital malignant mixed Mullerian tumor. Gynecol Oncol 1988; 30: 422–426. 3. Garde JR, Jones MA, McAfee R et al. Extragenital malignant mixed mullerian tumor: Review of the literature. Gynecol Oncol 1991; 43: 186–190. 4. Lauchlan SC. The secondary mullerian system revisited. Int J Gynecol Pathol 1994; 13 (1): 73–79. 5. Pirani M, Marcheselli R, Marcheselli L, Bari A, Federico M, Sacchi S. Risk for second malignancies in non-Hodgkin’s lymphoma survivors: A meta-analysis. Ann Oncol 2011; 22: 1845– 1858. 6. Gucer F, Pieber D, Arikan F. Malignancy arising in extraovarian endometriosis during estrogen stimulation. Eur J Gynaelcol Oncol 1998; 19: 39–41. 7. Lavery S, Gillmer M. Malignant transformation of residual endometriosis in women on unopposed oestrogen hormone replacement therapy. Br J Obstet Gynaecol 2001; 108: 1106– 1107.
© 2014 The Authors Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology
Retroperitoneal Müllerian tumor
8. Modesitt SC, Tortolero-Luna G, Robinson JB, Gershenson DM, Wolf JK. Ovarian and extraovarian endometriosisassociated cancer. Obstet Gynecol 2002; 100 (4): 788–795. 9. Heaps JM, Nieberg RK, Berek JS. Malignant neoplasms arising in endometriosis. Obstet Gynecol 1990; 75: 1023– 1028. 10. Han AC, Hovenden S, Rosenblum NG et al. Adenocarcinoma arising in extragonadal endometriosis: An immunohistochemical study. Cancer 1998; 83: 1163–1169. 11. Sampson JA. Endometrial carcinoma of the ovary, arising in endometrial tissue in that organ. Arch Surg 1925; 10: 1– 72.
12. Brooks JJ, Wheeler JE. Malignancy arising in extragonadal endometriosis. A case report and summary of the world literature. Cancer 1977; 40: 3065–3073. 13. Ulbright TM, Morley DJ, Roth LM, Berkow RL. Papillary serous carcinoma of the retroperitoneum. Am J Clin Pathol 1983; 79: 633–637. 14. Iura A, Sasajima Y, Katsumata N et al. Serous adenocarcinoma of the retroperitoneum, as a type of multifocal mullerian carcinoma. Int J Clin Oncol 2009; 14: 254– 257. 15. Roth LM, Ehrlich CE. Mucinous cystadenocarcinoma of the retroperitoneum. Obstet Gynecol 1977; 49: 486–488.
© 2014 The Authors Journal of Obstetrics and Gynaecology Research © 2014 Japan Society of Obstetrics and Gynecology
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